Pisa syndrome is a rare clinical entity characterized by marked lateral flexion of the trunk, which is typically mobile and resolves at supine position. When observed in clinical practice, it denotes an incapacitating symptom of underlying neurodegenerative disease such as Parkinson's disease, multiple system atrophy, and Alzheimer's disease. The underlying pathophysiological mechanisms responsible for the development of Pisa syndrome are poorly understood, and its management remains a challenge. In this review, we will focus our attention on Pisa syndrome in patients with Parkinson's disease, and provide an update on prevalence, pathophysiology, clinical manifestation, and treatment options.